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Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjögren syndrome

  • Immunology/Allergology
  • Published:
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Abstract

We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mononuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjögren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses.

Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.

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Authors and Affiliations

  1. Department of Internal Medicine, Universita' di Milano, Via Olgettina 60, 20132, Milano, Italy

    C. Besana, C. Salmaggi & C. Pellegrino

  2. Department of Ophthalmology, Universita' di Milano, Via Olgettina 60, 20132, Milano, Italy

    L. Pierro & S. Vergani

  3. Department of Pathology Istituto Scientifico San Raffaele, Universita' di Milano, Via Olgettina 60, 20132, Milano, Italy

    A. Faravelli & C. Rugarli

Authors
  1. C. Besana
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  2. C. Salmaggi
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  3. C. Pellegrino
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  4. L. Pierro
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  5. S. Vergani
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  6. A. Faravelli
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  7. C. Rugarli
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Besana, C., Salmaggi, C., Pellegrino, C. et al. Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjögren syndrome. Eur J Pediatr 150, 652–655 (1991). https://doi.org/10.1007/BF02072627

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  • DOI: https://doi.org/10.1007/BF02072627

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